Nonfluent/agrammatic PPA with in-vivo cortical amyloidosis and Pick's disease pathology

作者：Caso Francesca^*; Gesierich Benno; Henry Maya; Sidhu Manu; LaMarre Amanda; Babiak Miranda; Miller Bruce L; Rabinovici Gil D; Huang Eric J; Magnani Giuseppe; Filippi Massimo; Comi Giancarlo; Seeley William W; Gorno Tempini Maria Luisa

来源：Behavioural Neurology, 2013, 26(1-2): 95-106.

DOI：10.3233/BEN-2012-120255

• Nonfluent primary progressive aphasia
• PPA
• apraxia of speech
• Voxel-based morphometry
• PiB-PET
• Pick's disease
• Alzheimer disease
• Frontotemporal dementia

The role of biomarkers in predicting pathological findings in the frontotemporal dementia (FTD) clinical spectrum disorders is still being explored. We present comprehensive, prospective longitudinal data for a 66 year old, right-handed female who met current criteria for the nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA). She first presented with a 3-year history of progressive speech and language impairment mainly characterized by severe apraxia of speech. Neuropsychological and general motor functions remained relatively spared throughout the clinical course. Voxel-based morphometry (VBM) showed selective cortical atrophy of the left posterior inferior frontal gyms (IFG) and underlying insula that worsened overtime, extending along the left premotor strip. Five years after her first evaluation, she developed mild memory impairment and underwent PET-FDG and PiB scans that showed left frontal hypometabolism and cortical amyloidosis. Three years later (11 years from first symptom), post-mortem histopathological evaluation revealed Pick's disease, with severe degeneration of left IFG, mid-insula, and precentral gyms. Alzheimer's disease (AD) (CERAD frequent/Braak Stage V) was also detected. This patient demonstrates that biomarkers indicating brain amyloidosis should not be considered conclusive evidence that AD pathology accounts for a typical FTD clinical/anatomical syndrome.

• 出版日期2013